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Guidelines and parameters: percutaneous sclerotherapy for the treatment of head and neck venous and lymphatic malformations
  1. Jeremy J Heit1,
  2. Huy M Do2,
  3. Charles J Prestigiacomo3,
  4. Josser A Delgado-Almandoz4,
  5. Joey English5,
  6. Chirag D Gandhi6,
  7. Felipe C Albequerque7,
  8. Sandra Narayanan8,
  9. Kristine A Blackham9,
  10. Todd Abruzzo10,
  11. Barbara Albani11,
  12. Justin F Fraser12,
  13. Don V Heck13,
  14. M Shazam Hussain14,
  15. Seon-Kyu Lee15,
  16. Sameer A Ansari16,
  17. Steven W Hetts17,
  18. Ketan R Bulsara18,
  19. Michael Kelly19,
  20. Adam S Arthur20,
  21. Athos Patsalides21,
  22. G Lee Pride22,
  23. Ciaran J Powers23,
  24. Michael J Alexander24,
  25. Philip M Meyers25,
  26. Mahesh V Jayaraman26
  27. On behalf of the SNIS Standards and Guidelines committee
  1. 1Department of Radiology, Neuroadiology and Neurointervention Division, Stanford University, Stanford, California, USA
  2. 2Department of Radiology and Neurosurgery, Stanford University Medical Center, Stanford, California, USA
  3. 3Department of Neurological Surgery, New Jersey Medical School, University of Medicine and Dentistry of New Jersey, Newark, New Jersey, USA
  4. 4Department of Neurointerventional Radiology, Abbott Northwestern Hospital, Minneapolis, Minnesota, USA
  5. 5Department of Neurology and Radiology, UCSF, Stanford, California, USA
  6. 6Neurological Institute of New Jersey, New Jersey Medical School, Newark, New Jersey, USA
  7. 7Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Arizona, USA
  8. 8Department of Neurosurgery and Neurology, Wayne State University School of Medicine, Detroit, Michigan, USA
  9. 9University Hospitals Department of Radiology, Case Western Reserve University, Cleveland, Ohio, USA
  10. 10Department of Neurosurgery, University of Cincinnati, Cincinnati, Ohio, USA
  11. 11Department of Neurointerventional Surgery, Christiana Care Health Systems, Newark, Delaware, USA
  12. 12Department of Neurological Surgery, University of Kentucky, Lexington, Kentucky, USA
  13. 13Department of Radiology, Forsyth Medical Center, Winston Salem, North Carolina, USA
  14. 14Cerebrovascular Center, Cleveland Clinic, Cleveland, Ohio, USA
  15. 15Department of Radiology, University of Chicago, Chicago, Illinois, USA
  16. 16Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
  17. 17University of Medicine, Stanford, California, USA
  18. 18Department of Neurosurgery, Yale University School of Medicine, New Haven, Connecticut, USA
  19. 19Department of Neurosurgery, Royal University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
  20. 20Semmes-Murphey Neurologic and Spine Institute, Memphis, Tennessee, USA
  21. 21Department of Neurological Surgery, Weill Cornell Medical College, New York Presbyterian Hospital, New York, New York, USA
  22. 22Neuroradiology, UT Southwestern, Dallas, Texas, USA
  23. 23Department of Neurosurgery, Wexner Medical Center, Columbus, Ohio, USA
  24. 24Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, California, USA
  25. 25Radiology and Neurological Surgery, Columbia University, New York, New York, USA
  26. 26Warren Alpert School of Medical at Brown University, Providence, Rhode Island, USA
  1. Correspondence to Dr HM Do, Department of Radiology and Neurosurgery, S-047, Stanford University Medical Center, Stanford, CA 94305, USA; huymdo{at}stanford.edu

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Introduction

Vascular anomalies are classified broadly into vascular tumors and vascular malformations.1 Vascular malformations are further subdivided based on the histology of the lesion and whether the lesion is ‘high flow’ or ‘low flow’.1–3 The ‘low flow’ vascular anomalies are the focus of this article, and this category is broadly comprised of venous malformations (VM) and lymphatic malformations (LM). VM and LM are congenital anomalies thought to occur secondary to developmental errors in venous or lymphatic patterning, respectively.3–6 These lesions most commonly affect the head and neck, and have a prevalence of approximately 1%.3 ,5 ,7 Lesions involving the aerodigestive tract may result in airway obstruction, impaired food intake, and prevent normal speech. Similarly, lesions that involve the orbit may compromise visual acuity or ocular mobility. Vascular malformations may become quite large and demonstrate contiguous extension from the neck into the chest, pleura, cervical spine, or thoracic spine with associated pleural effusions and osteolysis. The vast majority of VM and LM are sporadic, although an increased prevalence of these anomalies has been described in multiple syndromes and with inherited gene mutations.8–13

It is important to recognize the categorization of LM into macrocystic or microcystic subtypes when contemplating treatment of these lesions. Macrocystic LM are comprised of larger cysts (≥2 cm in diameter) and respond well to percutaneous sclerotherapy and surgery with good to excellent outcomes in 76–95% of patients.3 ,8 ,14–17 By contrast, microcystic LM are comprised of innumerable small cysts (<2 cm in size) and tend to respond poorly to percutaneous sclerotherapy or surgery.3 ,8 ,18–20 Emerging data regarding the medical treatment of microcystic LM with sildenafil and the mTOR inhibitor sirolimus are promising, but these studies are still in an investigational stage.21 Effective treatment of superficial microcystic …

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